Sensitivity of current criteria for the diagnosis of behavioral variant frontotemporal dementia
Sensitivity of current criteria for the diagnosis of behavioral variant frontotemporal dementia
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DOI：
10.1212/01.wnl.0000343004.98599.45
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发表时间：
2009-02-24
期刊：
影响因子：
9.9
通讯作者：
Hodges, J. R.
中科院分区：
文献类型：
--
作者：
Piguet, O.;Hornberger, M.;Hodges, J. R.
背景：行为变异型额颞叶痴呆 (bvFTD) 的诊断依赖于具有约束性且可能不明确的标准。有些特征可供临床解释，但其普遍程度未知。本研究调查了当前诊断标准在一大群 bvFTD 患者中的敏感性。 方法：纳入了 45 名具有明显 bvFTD 证据的患者，根据临床进行性衰退（> 3 年）判断，具有明显的额叶特征和脑 MRI 上显着的额叶脑萎缩。三十二人死亡；所有 18 名进行尸检的患者均得到病理证实的额颞叶变性。我们确定了就诊时和疾病进展时核心和支持性诊断特征的患病率。结果：只有 25/45 名患者 (56%) 在初次就诊时表现出诊断 bvFTD 所需的全部五项核心特征，而随着疾病进展，这一比例为 33/45 (73%)。情绪迟钝和洞察力丧失这两个核心特征在 25% 和 13% 的案例中从未被观察到。执行功能障碍、多口、精神僵化和注意力分散是超过 50% 的病例在初次就诊时存在的唯一支持性特征。尽管不是诊断特征，但 33/45 名患者 (73%) 存在日常生活活动受损。结论：严格应用该标准会漏掉很大一部分患者。许多支持性特征的流行率较低并且在临床上没有用处。鼓励对标准进行修订，以包括取决于存在的特征数量和辅助信息（例如脑萎缩、神经心理异常、日常生活活动受损）的确定性水平（确定的、可能的、可能的）。神经病学（R）2009； 72：732-737
Background: Diagnosis of behavioral variant frontotemporal dementia (bvFTD) relies on criteria that are constraining and potentially ambiguous. Some features are open to clinical interpretation and their prevalence unknown. This study investigated the sensitivity of current diagnostic criteria in a large group of patients with bvFTD.Methods: Forty-five patients with clear evidence of bvFTD as judged by progressive clinical decline (> 3 years) with marked frontal features and significant frontal brain atrophy on brain MRI were included. Thirty-two have died; pathologic confirmation of frontotemporal lobar degeneration was found in all 18 coming to autopsy. We established the prevalence of core and supportive diagnostic features at presentation and with disease progression.Results: Only 25/45 patients (56%) showed all five core features necessary for a diagnosis of bvFTD at initial presentation and 33/45 (73%) as their disease progressed. Two core features, emotional blunting and loss of insight, were never observed in 25% and 13% of cases. Executive dysfunction, hyperorality, mental inflexibility, and distractibility were the only supportive features present in > 50% of cases at initial presentation. Although not a diagnostic feature, impaired activities of daily living was present in 33/45 patients (73%).Conclusions: Strict application of the criteria misses a significant proportion of patients. Many supportive features have low prevalence and are clinically not useful. Revision of the criteria to include level of certainty (definite, probable, possible) dependent on the number of features present and the presence of ancillary information (e. g., brain atrophy, neuropsychological abnormalities, impaired activities of daily living) is encouraged. Neurology (R) 2009; 72: 732-737